Faculty Bibliography

PURPOSE/OBJECTIVE:To describe a patient with progressive visual symptoms and reduced retinal sensitivity corresponding to dark without pressure (DWP). METHODS:Retrospective chart review of a single patient. Comprehensive ophthalmic examinations and multimodal imaging techniques, including optical coherence tomography (OCT), OCT-angiography, and microperimetry, were analyzed. RESULTS:A 23-year-old male presented with progressive peripheral areas of blurred vision superiorly in his right eye and temporally in his left eye. These disturbances corresponded with dark areas of retina inferiorly in his right eye and nasally in his left eye having characteristic features of DWP on multimodal imaging. Although Humphrey visual field (HVF) 24-2 testing was normal, microperimetry showed decreased retinal sensitivity in areas of DWP relative to adjacent areas without DWP. CONCLUSION/CONCLUSIONS:Prior descriptions of DWP have described it as a benign retinal finding showing no functional deficits. We demonstrate that DWP can be associated with progressive visual complaints showing decreased retinal sensitivity on microperimetry and undetected with HVF 24-2 testing.

PURPOSE/OBJECTIVE:To characterize and classify different forms of peripapillary retinoschisis (PPRS), and to clarify the nomenclature of this condition. METHODS:A retrospective, multicenter, multinational case series of PPRS was performed from August 2021 to September 2024. Cases were included if they demonstrated retinoschisis contiguous with and thought to be originating from the optic disc. Demographic and clinical data collected included age, gender, visual acuity, intraocular pressure, axial length, refraction and referring symptoms. Mandatory investigations included optical coherence tomography of the optic disc and macula with radial scans, colour fundus photography, and fundus autofluorescence. Select cases underwent fundus fluorescein and/or indocyanine green angiography. Retinoschisis was characterised by the meridian in relation to the optic disc, layer(s) of the retina affected and associated conditions. A literature review was performed to identify all causes of PPRS. RESULTS:A total of 47 eyes from 41 patients with PPRS were identified, comprising of 22 (54%) females and a mean age of 56 years (range 14-92 years). These were classified into nine aetiologies: Congenital Disc Abnormalities (CDA, n=16 eyes), peripapillary chorioretinal coloboma (n=1), peripapillary atrophy (n=3), glaucoma (n=7), Peripapillary Pachychoroid Syndrome (PPS, n=4), peripapillary choroidal neovascularization (PP-CNV, n=3), high/ pathological myopia (n=5), vitreopapillary traction (VPT, n=4) and idiopathic (n=4). CONCLUSION/CONCLUSIONS:The nine aetiologies of peripapillary retinoschisis can be classified into five groups: non-glaucomatous optic disc abnormalities (CDA, PP-coloboma, PP-atrophy), glaucomatous optic disc abnormalities, peripapillary choroidal diseases (PPS and PP-CNV), vitreous optic disc interface abnormalities and idiopathic. A new entity, "Focal Optic Disc Dome" (FODD) was identified. Understanding the full spectrum of PPRS can assist in correct diagnosis and management.

PURPOSE/OBJECTIVE:To establish consensus-based guidelines on the diagnosis, classification, and management of central serous chorioretinopathy (CSC) through a structured expert panel initiated by the Asia-Pacific Vitreo-retina Society (APVRS), the Academy of Asia-Pacific Professors of Ophthalmology (AAPPO), and the Academia Retina Internationalis (ARI), addressing the existing clinical controversies. METHODS:An international panel of 26 experts from 13 countries collaboratively drafted consensus statements spanning five key areas: disease definition, pathophysiology, investigations, current management, and future developments. Consensus was reached through an iterative Delphi process and anonymous voting using a five-point Likert scale. Statements were accepted when >75 % agreement ('agree' & 'strongly agree') was achieved. RESULTS:Consensus was achieved for all 25 statements, reflecting strong alignment among experts. Key agreements included defining CSC as a pachychoroid-driven chorioretinal disorder characterized by neurosensory retinal and/or RPE detachment, with multimodal imaging (optical coherence tomography, fundus autofluorescence, fluorescein angiography, and indocyanine green angiography) recognized as essential for diagnosis. Half-dose photodynamic therapy (PDT) was unanimously endorsed as the first-line treatment for chronic CSC. Oral mineralocorticoid receptor antagonists (MRAs) lacked consensus for therapeutic benefit, aligning with evidence from the VICI and SPECTRA trials. Anti-vascular endothelial growth factor receptor therapy was recommended solely for CSC complicated by a macular neovascularization. Future priorities highlighted standardizing disease classification and exploring targeted therapies through genetic and nanomedicine research. CONCLUSION/CONCLUSIONS:This consensus initiative provides a robust, evidence-based framework for the diagnosis and management of CSC, promoting standardization across clinical practices and guiding future research directions to address persistent gaps in CSC care.

PURPOSE/UNASSIGNED:To investigate whether consensus can be reached on the acceptability of end-stage atrophy onset as a clinical end point in early intervention trials of age-related macular degeneration (AMD), and the criteria for defining such an end point. DESIGN/UNASSIGNED:A modified Delphi study. PARTICIPANTS/UNASSIGNED:International panel of experts in AMD, retinal imaging, and histopathology that are part of the Classification of Atrophy Meetings group. METHODS/UNASSIGNED:A modified Delphi study was undertaken to determine if there is consensus on the acceptability of the onset of end-stage atrophic AMD as a clinical end point to evaluate early interventions and the criteria for defining such an end point. Two initial rounds of online surveys were conducted. Aggregate results and anonymized comments were provided after each round, followed by a face-to-face meeting before a final survey round was completed. MAIN OUTCOME MEASURES/UNASSIGNED:Statements where consensus was reached, defined as ≥80% of responses within the 3-point bracket for agreement or disagreement based on a 9-point Likert rating scale, from a total of 33 statements included in the final round of the survey. RESULTS/UNASSIGNED:Consensus was reached for the statement that the onset of end-stage atrophic AMD was an appropriate clinical end point to evaluate early interventions (82% responses in agreement). Consensus was also reached for the statement that such an end point should be defined based on anatomical changes that have been previously shown in clinical studies to be associated with marked, but not necessarily complete, functional loss (95% responses in agreement). Consensus was nearly reached for the specific criterion that ≥90% of such atrophic AMD lesions should have ≥1 test location that was ≤10 decibels on 2 microperimetry tests (77% responses in agreement). CONCLUSIONS/UNASSIGNED:There was expert group consensus that the onset of end-stage atrophy is an appropriate clinical end point to evaluate early interventions in AMD, and that such an end point should show evidence of marked functional loss in prior clinical studies. We believe these findings will help to define incident clinical end points that are acceptable to regulatory authorities. FINANCIAL DISCLOSURES/UNASSIGNED:Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

PURPOSE/OBJECTIVE:To explore the impact of home optical coherence tomography (OCT)-guided treatment personalization for type 3 macular neovascularization (MNV) in age-related macular degeneration (AMD). METHODS:A prospective home OCT trial of a patient with a one-month history of type 3 MNV under anti-angiogenic treatment (two "loading" injections). During the 23.4-week study period, the patient's treatment regimen was managed through regular self-imaging, enabling continuous monitoring of retinal fluid dynamics. RESULTS:Over the observation period, the patient performed 143 home OCT measurements (mean 6.1 per week, standard deviation 1), which prompted three in-office visits where three anti-angiogenic injections were delivered at the discretion of the investigator. The mean individualized treatment intervals established through home OCT were 8 weeks, with the patient maintaining stable visual acuity at 20/20 Snellen. Complete resolution of intraretinal fluid was noted between injection. Notably, home OCT revealed unexpected fluctuations in retinal fluid measured as hyporeflective spaces in the absence of treatment. Two out of three injections were administered after the retinal fluid volume had decreased by more than half within two, and eight days following an initial transient spike. CONCLUSION/CONCLUSIONS:The neovascular subtype as defined by the Consensus Nomenclature for Reporting Neovascular AMD Data (CONAN) may serve as a useful management framework when approaching home OCT guided treatment decisions. In type 3 disease, home OCT-guided management demonstrates significant potential for optimizing treatment. Further research is warranted to elucidate the dynamics of retinal fluid variations in different MNV subtypes.

PURPOSE/OBJECTIVE:To develop imaging and consensus-based guidelines on the application of multimodal imaging in multiple evanescent white dot syndrome (MEWDS). DESIGN/METHODS:Consensus agreement guided by literature, and an expert committee using a nominal group technique (NGT). METHODS:The expert committee employed a structured NGT with multiple rounds of discussion, conflict resolution, and anonymous voting to: (1) establish imaging criteria for diagnosing and monitoring MEWDS using color fundus photography (CFP), optical coherence tomography (OCT), fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA); and (2) develop consensus-based recommendations for assessing specific characteristics in patients with MEWDS. These formal recommendations were derived from a structured NGT using illustrative cases of MEWDS and were further voted upon by the entire task force. RESULTS:The diagnosis of acute MEWDS is supported by distinct multimodal features on CFP, multi-focal disruption of the ellipsoid/interdigitation zone with overlying outer retinal hyper-reflectivity with OCT, and hyper-autofluorescent spots with FAF (short-wave blue/green). In complex cases, wreath-like lesions on FFA and the absence of early hypofluorescence on ICGA help differentiate MEWDS from other chorioretinopathies. The lack of specific choroidal changes on OCT and preserved signal on OCTA on retinal and inner choroidal slabs also aid in diagnosis. CONCLUSIONS:Multimodal imaging is essential for diagnosing MEWDS and differentiating it from other non-infectious uveitis types, extending the Standardization of Uveitis Nomenclature (SUN) classification. These imaging criteria enable detailed assessment of disease activity and offer valuable insights into MEWDS pathogenesis.

PURPOSE/OBJECTIVE:To develop consensus nomenclature amongst international retinal specialists for the distinctive optical coherence tomography (OCT) finding of a lesion originating from the retinal capillary bed, measuring ≥100 µm in size, and characterized by a hyperreflective wall with a hyporeflective lumen. METHODS:A comprehensive literature search was performed from inception to January 2024 on three databases to elicit publications reporting on relevant vascular abnormalities and corresponding nomenclature. A panel of retinal specialists with expertise in this topic reviewed the list of candidate terms and proposed other names for the lesion of interest. A refined list was then incorporated into a Delphi survey, which was distributed to the general membership of the International Retinal Imaging Society (IntRIS). Consensus was defined as at least 70% agreement amongst participants. RESULTS:An expert panel (n=11) reviewed candidate names for the lesion, with poor agreement noted amongst panel members regarding the relevant nomenclature. In the first Delphi survey, (n=70 IntRIS members), the need for a unified nomenclature was highlighted and two leading candidate names were established: large retinal capillary aneurysm (LRCA, n=38, 54.3%) and retinal capillary macroaneurysm (n=14, 20.0%). A second follow-up survey (n=54 IntRIS members) established LRCA (n=44, 81.5%) as the consensus term to identify the OCT vascular abnormality. CONCLUSION/CONCLUSIONS:This Delphi project reached consensus on a unifying term, large retinal capillary aneurysm, for a specific and signature OCT lesion. Identification of this characteristic OCT finding and adoption of this term may facilitate diagnosis, guide therapeutic decisions, and improve clinical and scientific communication.

PURPOSE/OBJECTIVE:To describe the clinical features, multimodal imaging findings, natural history, and treatment outcomes of acute outer retinopathy (AOR), which represents an expanded spectrum of acute annular outer retinopathy (AAOR). DESIGN/METHODS:Retrospective, observational, longitudinal, multicenter case series. PARTICIPANTS/METHODS:Twenty-three patients (15 female, 8 male) with a mean age of 41.8 ± 18.6 years (range: 14-86 years) and a mean follow-up duration of 3.7 ± 1.5 years (range: 1-12 years). METHODS:Clinical characteristics, multimodal imaging findings, laboratory evaluations, genetic testing, natural history, therapeutic management, and outcomes were reviewed and analyzed. MAIN OUTCOMES MEASURES/METHODS:Specific multimodal imaging signatures of AOR were identified, including findings from ophthalmoscopy, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). Humphrey visual field testing, full-field electroretinography (ERG), and multifocal ERG were analyzed. Baseline features and the natural course of the disease were delineated. RESULTS:Thirty-eight eyes from 23 patients were analyzed. Presenting symptoms included photopsia (87%), blurred vision (57%), and scotoma (57%). On ophthalmoscopy, AOR was acutely characterized by yellow-greyish outer retinal lesions corresponding to hyperautofluorescent changes on FAF and the angular sign of Henle fiber layer hyperreflectivity (ASHH) on OCT. FAF imaging revealed ring-like hyperautofluorescent lesions surrounding the optic disc in 18% of eyes. Additional lesion patterns on FAF included perivenular (53%), sectoral (16%), and spot-like distributions (13%). FA and ICGA findings were mostly unremarkable. Lesion progression primarily occurred within the initial weeks following presentation and stabilized in size beyond this period in the majority of eyes. Over time, affected areas progressed to outer retinal atrophy with pigmentary changes. Foveal sparing was observed in 68% of the eyes. None of the therapeutic interventions appeared effective in halting the progression to complete outer retinal atrophy or preventing lesion enlargement. CONCLUSIONS:AOR is characterized by early photoreceptor disruption, evidenced by ASHH on OCT, leading to rapid outer retinal atrophy and subsequent degeneration of the retinal pigment epithelium within the damaged zones. Although distinct patterns of lesion distribution were observed, their consistent features on multimodal imaging support their inclusion within a unified disease spectrum termed acute outer retinopathy.

PURPOSE/OBJECTIVE:To characterize the clinical and multimodal imaging features of central bouquet hemorrhage (CBH) with Henle fiber layer (HFL) involvement in highly myopic eyes, and to investigate the relationships between hemorrhage characteristics, reabsorption time, and visual outcomes. METHODS:Multicenter, retrospective analysis of highly myopic eyes with CBH involving the HFL, confirmed by optical coherence tomography (OCT). RESULTS:Eighteen eyes from 18 subjects were included for analysis. The mean age of the cohort was 39 ± 13.7 years (range: 17-69) and 61% of subjects were female. Mean refractive error was -14.8 ± 3.14 diopters (range: -9 D to -22 D). All eyes demonstrated a combined CBH with HFL component, while a subretinal component was present in 83.3% of cases. Myopic choroidal neovascularization (CNV) was excluded in all eyes using optical coherence tomography angiography (OCTA) or dye-based angiography (fluorescein or indocyanine green). No correlation was observed between hemorrhage size and visual outcomes or reabsorption time. Hemorrhage cleared after a mean of 2.63 months, and the radial HFL hemorrhage component resolved first. All eyes showed improvement in visual acuity from baseline. Persistent OCT alternations after resolution of hemorrhage included ellipsoid zone disruption (88.9%) and hyperreflective changes in HFL (77.8%). Anti-VEGF injections were administered to 6 eyes (33.3%) and did not correlate with a significant visual or anatomical benefit. CONCLUSION/CONCLUSIONS:CBH with HFL involvement in high myopia was associated with significantly improved visual outcomes from baseline but structural alterations can persist after clinical resolution. The size of the hemorrhage did not correlate with resorption time, and anti-VEGF treatment did not affect outcome. These findings provide new insights into the natural history and management of nonneovascular CBH in highly myopic eyes.